Appendiceal Neuroendocrine Tumor Is a Rare Cause of Ectopic Adrenocorticotropic Hormone Syndrome With Cyclic Hypercortisolism: A Case Report and Literature Review

Overview

Journal Front Endocrinol (Lausanne)

Specialty Endocrinology

Date 2022 Mar 7

PMID 35250866

Authors

Yu Xing Zhao

Wan Lu Ma

Yan Jiang

Guan Nan Zhang

Lin Jie Wang

Feng Ying Gong

Hui Juan Zhu

Lin Lu

Affiliations

Soon will be listed here.

Abstract

Objective: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a condition of hypercortisolism caused by non-pituitary tumors secreting ACTH. Appendiceal neuroendocrine tumor as a rare cause of ectopic ACTH syndrome was reported scarcely. We aimed to report a patient diagnosed with EAS caused by an appendiceal neuroendocrine tumor and summarized characteristics of these similar cases reported before.

Case Report And Literature Review: We reported a case with Cushing's syndrome who was misdiagnosed as pituitary ACTH adenoma at first and accepted sella exploration. Serum and urinary cortisol decreased, and symptoms were relieved in the following 4 months after surgery but recurred 6 months after surgery. The abnormal rhythm of plasma cortisol and ACTH presented periodic secretion and seemingly rose significantly after food intake. EAS was diagnosed according to inferior petrosal sinus sampling (IPSS). Appendiceal mass was identified by Ga-DOTA-Tyr3-octreotate (DOTATATE)-PET-CT and removed. The pathological result was consistent with appendiceal neuroendocrine tumor with ACTH (+). The literature review demonstrated 7 cases diagnosed with EAS caused by appendiceal neuroendocrine tumor with similarities and differences.

Conclusion: The diagnosis of an ectopic ACTH-producing tumor caused by the appendiceal neuroendocrine tumor can be a challenging procedure. Periodic ACTH and cortisol secretion may lead to missed diagnosis and misdiagnosis. IPSS is crucial in the diagnosis of EAS and Ga-DOTATATE-PET-CT plays an important role in the identification of lesions.

Citing Articles

ACTH-Independent Cushing's Syndrome Caused by an Ectopic Adrenocortical Adenoma in the Renal Hilum.

Hao Z, Ding J, Huo L, Luo Y Diagnostics (Basel). 2022; 12(8).

PMID: 36010286 PMC: 9407005. DOI: 10.3390/diagnostics12081937.

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